Pulmonary Embolism

(PE)

Pulmonary embolism is the blocking of an artery of the lung (pulmonary artery) by a collection of solid material brought through the bloodstream (embolus)—usually a blood clot (thrombus) or rarely other material.

Pulmonary embolism is usually caused by a blood clot, although other substances can also form emboli and block an artery.

Symptoms of pulmonary embolism vary but usually include shortness of breath.

Doctors often diagnose pulmonary embolism by looking for blockage of a pulmonary artery using computed tomography (CT) angiography or lung scanning.

Blood thinners (anticoagulant drugs) can be given to people at high risk to prevent pulmonary embolism.

Anticoagulant drugs are used to keep emboli from enlarging while the body dissolves the clots; other measures (such as drugs to break up blood clots or surgery) may be needed for people who appear to be at risk of dying.

The pulmonary arteries carry blood from the heart to the lungs. The blood picks up oxygen from the lungs and travels back to the heart. From the heart, the blood is pumped to the rest of the body to Home. provide oxygen to the tissues. When a pulmonary artery is blocked by an embolus, people may not be able to get sufficient oxygen into the blood. Large emboli may cause so much blockage that the heart has to strain to pump blood through the pulmonary arteries that remain open (massive pulmonary embolism). If too little blood is pumped or the heart is strained excessively, the person can go into shock and die. Sometimes, the blockage of blood flow causes lung tissue to die (a condition called pulmonary infarction).

The body usually breaks up small clots more quickly than larger clots, keeping damage to a minimum. Large clots take much longer to disintegrate, so more damage may be done.

Pulmonary embolism affects about 350,000 people per year and causes 85,000 deaths per year in the United States. It affects mainly adults.

Causes

The most common type of pulmonary embolism is a

Blood clot

Usually the Home. blood clot forms in a leg or pelvic vein when blood flow slows down or stops, as may occur in the leg veins when a person stays in one position for a long time after an injury (for example, a hip fracture) or major surgery. Other causes include conditions that make the blood clot more easily or the presence of a foreign substance within the bloodstream (for example, an intravenous catheter).

What Predisposes Someone to Blood Clots?

The cause of blood clots in the veins may not be discernible, but many times predisposing conditions (risk factors) are obvious. These conditions include

Catheters inserted into a large vein for giving drugs or nutrients (indwelling venous catheters)

Heart failure

Immobility

Injury to the pelvis, hip, or a leg

A kidney disorder called nephrotic syndrome

Major surgery within the past 3 months

Disorders of the bone marrow that make the blood too thick (hyperviscosity)

Obesity

Pregnancy or the period after delivery

Prior blood clot

Sickle cell disease

Smoking

Stroke

Use of estrogens, for example, as treatment for menopausal symptoms or as contraception (in which case the risk is particularly high among women who are older than 35 or who smoke)

Use of estrogen receptor modulators (such as raloxifene or tamoxifen)

Use of testosterone replacement therapy

People who sit for long time periods without moving around (as may happen during air travel) are at slightly increased risk.

Far less often, blood clots form in the veins of the arms or in the right side of the heart. Once a clot breaks free into the bloodstream, it usually travels to the lungs.

Unusual types of emboli

The sudden blocking of an artery of the lung is not only caused by blood clots. Other material can also form emboli.

Fat can escape into the blood from the bone marrow when a long bone is fractured or during bone surgery and form an embolus.

Amniotic fluid that is forced into the pelvic veins during a complicated childbirth can form an embolus.

Cancer cells in clumps may break free into the circulation to form tumor emboli.

Air bubbles may form emboli if a catheter in one of the large veins (central veins) is inadvertently opened to air. Air emboli may also form when a vein is operated on (such as when a blood clot is being removed). An additional risk is underwater diving (Home. decompression sickness).

Infected material may also form emboli and travel to the lung. Causes include intravenous drug use, certain heart valve infections, and inflammation of a vein with blood clot formation and infection (septic thrombophlebitis).

A foreign substance can be introduced into the bloodstream, usually by intravenous injection of inorganic substances such as talc or mercury by injection drug users, where it can form emboli and travel to the lung.

Small emboli may not cause any symptoms, but when symptoms do occur, they often develop abruptly.

Symptoms of pulmonary embolism may include

Shortness of breath

Chest pain

Light-headedness or fainting

Shortness of breath may be the only symptom, especially if pulmonary infarction does not develop. Often, the breathing is very rapid, and the person may feel anxious or restless and appear to have an anxiety attack.

Some people have pain in the chest. The heartbeat may become rapid, irregular, or both.

In some people, particularly those with very large emboli, the first pulmonary embolism symptoms are light-headedness or loss of consciousness. If people suddenly lose consciousness, their body may shake, appearing to be seizures. Blood pressure may drop dangerously low (a condition called Home. shock), the skin may be cool and have a blue color (cyanosis), and the person could suddenly die.

In older people, the first symptom of pulmonary embolism may be confusion or deterioration of mental function. These symptoms usually result from a sudden decrease in the heart’s ability to deliver enough oxygen-rich blood to the brain and other organs.

Pulmonary infarction

Pulmonary infarction is when some of the lung tissue dies due to blockage of a lung blood vessel by a pulmonary embolus. Usually the embolus causing pulmonary infarction is small. The symptoms of pulmonary infarction develop over hours. If pulmonary infarction occurs, the person experiences coughing that may produce blood-stained sputum, sharp chest pain when breathing in, and in some cases fever. Symptoms of infarction often last several days but usually become milder every day.

Recurring emboli

In people who have recurring episodes of small pulmonary emboli, blood pressure in the lungs' blood vessels can increase. This can cause symptoms, such as shortness of breath, swelling of the ankles or legs, and weakness, which tend to develop progressively over weeks, months, or years.

Diagnosis

Pulse oximetry and chest x-ray

CT angiography, ultrasonography of the legs, lung perfusion scan, or a combination

Doctors suspect pulmonary embolism based on the person’s symptoms and risk factors, such as recent surgery, a prolonged period of bed rest, or a tendency to form blood clots. A large pulmonary embolism may be relatively easy for doctors to diagnose, especially when the person has obvious conditions that could cause pulmonary embolism, such as signs of a blood clot in a leg. However, in many cases, symptoms are absent or unusual, which is an important reason why pulmonary embolism is often difficult to diagnose. Indeed, pulmonary embolism is one of the most difficult serious disorders for doctors to recognize and diagnose.

Some routine tests can provide clues that a pulmonary embolism has occurred. However, these tests cannot diagnose with certainty whether a pulmonary embolism is truly present.

Tests that suggest pulmonary embolism

A Home. chest x-ray may reveal subtle changes in the blood vessel patterns that occur after embolism and may reveal signs of pulmonary infarction. However, the x‑ray results are often normal, and even when they are abnormal, they rarely enable doctors to establish the diagnosis with certainty.

Home. Electrocardiography (ECG) may show abnormalities. These abnormalities can support or suggest the diagnosis of pulmonary embolism but cannot confirm it.

The level of oxygen in the blood is measured with a sensor that is attached to a fingertip (oximetry). Because pulmonary embolism blocks pulmonary arteries, the level of oxygen in the blood may be low.

Doctors first judge how likely pulmonary embolism seems to be, based on information such as the person's risk for pulmonary embolism, the severity of their symptoms, and the results of early tests (such as the chest x-ray and level of oxygen in the blood).

If pulmonary embolism seems unlikely, a blood test that measures a substance called d‑dimer is typically done. This test may be the only test needed in such people. If in these people the d-dimer level is normal, then the probability that a pulmonary embolus has occurred is extremely low. However, although a low level of D-dimer in such people means that pulmonary embolism is unlikely, a high level does not necessarily mean that pulmonary embolism is likely. Other disorders, such as infection or injury, can cause the d-dimer level to be high so additional testing is needed to confirm the diagnosis.

If pulmonary embolism seems more likely or if the result of the d-dimer test is abnormal, other testing is done, usually one or more of the following:

Ultrasonography of the legs is noninvasive and can identify clots in the legs, which are the usual sources of pulmonary embolism. The absence of clots on this test does not rule out pulmonary embolism. However, if ultrasonography reveals blood clots, people are usually treated as they would be for pulmonary embolism without any further testing, because the treatment for both conditions is the same.

Tests to diagnose pulmonary embolism

CT angiography is a type of CT scan. It is fast, noninvasive, and fairly accurate, particularly for large clots. In this test, contrast material is injected into a vein. The contrast material travels to the lungs, and a CT scanner generates images of blood in the arteries to determine if a pulmonary embolism is blocking blood flow. CT angiography is the imaging test most often used to diagnose pulmonary embolism.

A Home. lung perfusion scan is noninvasive and fairly accurate but takes longer than a CT scan. A tiny amount of radioactive substance is injected into a vein and travels to the lungs, where it outlines the blood supply (perfusion) of the lung. Completely normal scan results usually indicate that the person does not have a significant blood vessel obstruction. Abnormal scan results support the possibility of pulmonary embolism but may also indicate the possibility of other disorders, such as emphysema, which can result in decreased blood flow to areas where lung tissue has been damaged.

Usually, the perfusion scan is done with a lung ventilation scan. In this test, the person inhales a harmless gas containing a trace amount of radioactive material, which is distributed throughout the small air sacs of the lungs (alveoli). The areas where carbon dioxide is being released and oxygen is taken up can then be seen on a scanner. By comparing this scan to the pattern of blood supply shown on the perfusion scan, doctors can usually determine whether a person has had a pulmonary embolism. Doctors sometimes use a lung perfusion scan if a person has a kidney problem that prevents the use of CT angiography because the contrast material used for the CT might further damage the kidneys.

Tests for serious or recurring emboli

Echocardiography may show that a blood clot is in the right atrium or right ventricle of the heart. The results of this test may help doctors determine the severity of the embolism by showing that the right side of the heart is strained by trying to push blood through the clots.

In people who have recurring blood clots, doctors may also measure proteins in the blood to determine whether a clotting disorder is the cause.

Prognosis

The likelihood of dying from pulmonary embolism is very low, but massive pulmonary embolism can cause sudden death. Most deaths occur before the diagnosis is suspected, often within a few hours of the embolism occurring. Factors important in determining the prognosis include

The size of the embolus

The size of the pulmonary arteries blocked

The number of pulmonary arteries blocked

The effect on the heart's ability to pump blood

The person’s overall health status

Anyone with a serious heart or lung problem is at greater risk of dying due to a pulmonary embolism. A person with normal heart and lung function usually survives unless the embolus blocks half or more of the pulmonary arteries.

Did You Know...

Pulmonary embolism is one of the most common causes of unexplained deaths.

Prevention

Given the danger of pulmonary embolism and the limitations of treatment, doctors try to prevent blood clots from forming in the veins of people at risk. In general, people, particularly those who are prone to clotting, should try to be active and move around as much as possible. For example, when traveling on an airplane for a long period, people should try to get up and move around every 2 hours.

Anticoagulation for pulmonary embolism

For certain people, an anticoagulant drug (also called a blood thinner) is given, most often heparin.

Heparin comes in two forms:

Traditional

Low molecular weight

Traditional and low molecular weight heparins appear equally effective. Heparin is the most widely used drug for reducing the likelihood of clots forming in calf veins after any type of major surgery, especially surgery on the legs. Small doses are injected just under the skin, usually within 6 to 12 hours after surgery, and ideally additional doses are given until the person is up and walking again.

Hospitalized people at high risk of developing pulmonary embolism (such as those with heart failure, immobility, or obesity, or who have had clots in the past) benefit from small doses of heparin even if they are not undergoing surgery. Low-dose heparin does not increase the frequency of severe bleeding complications, but heparin can increase minor oozing of blood from wounds.

Warfarin, an anticoagulant given by mouth, may be given to people with one or more risk factors. It is also given to people who have undergone certain kinds of surgery that are particularly likely to result in clots, such as surgery for a hip fracture or a joint replacement. Warfarin therapy may need to be continued for several weeks or months. Low-molecular-weight heparin is also effective for people in this situation.

Newer anticoagulants include those such as fondaparinux, rivaroxaban, apixaban, edoxaban, and dabigatran that inhibit the formation of substances that enhance the body’s production of clots. These drugs are effective in prevention and in some cases may be safer than warfarin.

Physical measures

For people who have undergone surgery—especially older people—the risk of clot formation can be reduced by

Using intermittent air compression devices or wearing compression elastic stockings

Doing leg exercises

Getting out of bed and becoming active as soon as possible

Intermittent air compression devices can provide rhythmic external pressure to keep blood moving in the legs. However, these devices alone are inadequate to prevent clot formation in people who have undergone hip or knee surgery.

In patients who are at high risk of developing pulmonary embolism and who cannot take anticoagulants due to a high risk of bleeding, a filter (called an inferior vena cava filter) may be placed inside a large vein between the heart and the inferior vena cava, which returns blood to the heart from the lower part of the body. A filter can trap emboli, preventing them from reaching the lungs.

Inferior Vena Cava Filters: One Way to Prevent Pulmonary Embolism

To prevent pulmonary embolism, doctors usually use drugs that limit blood clotting. However, for some people, doctors may recommend that a filter (formerly called an umbrella) be temporarily or permanently placed in the inferior vena cava. This filter device typically is recommended when drugs that limit clotting cannot be used, for example, when a person is also having bleeding. The filter can trap emboli before they reach the heart but allow blood to flow through freely. Emboli that are trapped sometimes dissolve on their own.

Treatment

Supportive therapy

Anticoagulation

Sometimes placement of an inferior vena cava filter

Sometimes thrombolytic ("clot busting") therapy

Pulmonary embolism treatment begins with treating the symptoms. Oxygen is given if blood oxygen levels are low. Analgesics are given to relieve pain. If blood pressure is low, intravenous fluids are given and sometimes drugs that increase blood pressure are given. Mechanical ventilation (a breathing tube) may be needed if respiratory failure develops.

Anticoagulation

Anticoagulant drugs are given to prevent existing blood clots from enlarging and additional clots from forming. Drug options include warfarin or newer anticoagulants, such as apixaban, rivaroxaban, edoxaban, and dabigatran. However, when edoxaban or dabigatran is used, doctors must give heparin therapy (by vein or by injection under the skin) for the first 5 to 10 days of therapy before they can give the edoxaban or dabigatran, which sometimes means the person must remain in the hospital. When warfarin therapy is selected, both heparin and warfarin are given for the first 5 to 10 days of therapy, and then warfarin alone is used thereafter.

Warfarin therapy requires periodic blood testing to ensure that the blood is thin enough to prevent clots from forming but not so thin as to cause a bleeding tendency (called excessive anticoagulation). The warfarin dose is frequently adjusted based on the results of the blood tests. Also, warfarin interacts with many different types of food and with other drugs, which can result in blood that is too thin or too thick. If excessive anticoagulation occurs, severe bleeding in a number of body organs can develop.

Because many drugs can interact with warfarin, people who take anticoagulants should be sure to check with their doctor before taking any other drugs, including drugs that can be obtained without a prescription (over-the-counter drugs) such as acetaminophen or aspirin, herbal preparations, and dietary supplements. Foods that are high in vitamin K (which affects blood clotting), such as broccoli, spinach, kale, and other leafy green vegetables, liver, grapefruit and grapefruit juice, and green tea, may need to be either eaten in very consistent amounts or avoided.

The newer anticoagulants, such as apixaban, rivaroxaban, edoxaban, and dabigatran, have several advantages over heparin or warfarin. Like heparin, these drugs can be taken by mouth, but dose adjustments and tests to monitor the level of anticoagulation are not needed. Furthermore, these drugs do not often interact with food or other drugs and are less likely to cause severe types of bleeding when compared with warfarin. Rivaroxaban should always be taken with food.

How long anticoagulants are given depends on the person’s situation. If pulmonary embolism is caused by a temporary risk factor, such as surgery, treatment is given for 3 months. If the cause is some longer-term problem, such as prolonged bed rest, treatment usually is given for 6 to 12 months, but sometimes it must continue indefinitely. For example, people who have recurrent pulmonary embolism, often because of a hereditary clotting disorder or cancer, usually take anticoagulants indefinitely.

Thrombolytic therapy

Thrombolytic drugs ("clot busting drugs") such as streptokinase or alteplase break up and dissolve blood clots. Because these drugs can cause dangerous or fatal bleeding, they are usually only used in people who appear to be in danger of dying due to the pulmonary embolism. Except in the most dire situations, these drugs are usually not be given to people who have had surgery in the preceding 2 weeks, are pregnant, have had a recent stroke, or tend to bleed excessively.

Physical measures

In some centers, if a person appears to be in danger of dying from a massive pulmonary embolism, doctors may try to break up the embolus using a catheter inserted into the pulmonary artery. Surgery may be needed in some cases of severe embolism. Removal of the embolus from the pulmonary artery may be lifesaving. Surgery is also used to remove long-standing pulmonary artery clots that cause persistent shortness of breath and high pressures in the pulmonary artery (Home. pulmonary hypertension).

A Home. filter can be surgically placed in the main vein in the abdomen that drains blood from the legs and pelvis to the right side of the heart. Such a filter can be used if emboli recur despite anticoagulant treatment or if anticoagulants cannot be used or cause significant bleeding. Because clots generally originate in the legs or pelvis, this filter usually prevents them from being carried into the pulmonary artery. Newer filters are removable. Removal helps prevent some complications that can occur when filters are left in place permanently.

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In autoimmune disorders that involve the lungs, a person's immune system mistakenly attacks and damages the person's own lung tissues. Which of the following conditions is an example of an autoimmune disorder of the lungs?

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